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Familial Mediterranean Fever (FMF) is a disease characterized by attacks and colchicine is the medication considered most effective in reducing the intensity and frequency of attacks. Adherence to the medication regiment is important not only to manage FMF symptoms, but also to prevent amyloidosis.

Results The mean age of disease onset was 5.1 ± 3.6 years and the mean age at diagnosis was 7.3 ±3.1 years. The mean number of attacks per year was 7.7 ± 7.8, the mean duration of attacks 1.7 ± 1.6 days. The most common clinical manifestations were fever (91.6%) and abdominal pain (90.3%). The other manifestations were arthralgia (44.1%), arthritis (18.6%), headache (17.3%), myalgia (15.6%), vomiting (11%), chest pain (6.8%), splenomegaly (5.5%), diarrhea (5.5%) and erisipelas like eritema (3.4%). Amyloidosis (1.8%) and proteinuria (1.8%) were also determined. Most of patients had compound heterozygote genotype (n=86, 28.6%) and the most common homozygote mutation was M694V homozygosity (n=65, %21.6). The frequency of headache in the patients with homozygote M694V mutation (n=18, %29.5) and the frequencies of arthritis in patients with homozygote E148Q (n=1, %33.3) and M694V mutations (n=16, % 26.2) were significantly higher than the patients with other mutations (p

The patients characterized with chronic subclinical inflammation even during attack-free periods, are now considered to have an increased risk of atherosclerotic complications as well as other autoinflammatory disease. Damage to the arterial wall due to atherosclerosis causes increased arterial stiffness. Pulse wave velocity (PWV), a noninvasive measure of arterial stiffness, is accepted to be an indicator of subclinical atherosclerosis. Cardiovascular disease included various risk markers; blood biomarkers and genetic markers. Matrix metalloproteinases (MMPs) are closely related proteinases that together are able to degrade all macromolecules of the extracellular matrix. MMPs are potentially implicated in atherogenesis, progression of atherosclerosis. The gene encoding MMP-3 is polymorphic and an insertion (6A)/deletion (5A) polymorphism (5A/6A polymorphism) in the MMP-3 gene may have functional significance in the regulation of its expression. The 5A allele was associated with higher and the 6A allele with lower transcriptional activity. Up to date, the 6A/6A and 5A/6A genotypes were associated with coronary artery disease and carotid atherosclerosis in adults.

Familial Mediterranean Fever (FMF) is a disease characterized by attacks and colchicine is the medication considered most effective in reducing the intensity and frequency of attacks. Adherence to the medication regiment is important not only to manage FMF symptoms, but also to prevent amyloidosis.

Objective Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent attacks of fever and peritonitis, pleuritis, arthritis or an erysipelaslike skin disorder. The disease may present at any age, more than 80% of patients being symptomatic by the age of 20 yr. Its main long-term complication is amyloid A (AA) amyloidosis, a severe manifestation with poor prognosis. Mutations in the MEFV gene, on chromosome 16, is encoding a protein named as pyrin. We aim to analyze clinical characteristics, subclinical inflammation, and carried MEFV mutation in siblings with FMF.

An increased incidence of cardiovascular disease has been found in rheumatic disorders. Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in children. Prolonged immunological inflammatory process leads in these patients to an early onset of atherosclerosis.